Paroxysmal nocturnal hemoglobinuria treated with eculizumab in Wakayama, Japan: a retrospective analysis

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  • TAMURA Shinobu
    Department of Hematology/Oncology, Wakayama Medical University Department of Internal Medicine, Kainan Municipal Medical Center
  • FURUYA Yoshiaki
    Department of Hematology/Oncology, Wakayama Medical University Department of Hematology, Kinan Hospital
  • HORI Yoshikazu
    Department of Hematology/Oncology, Wakayama Medical University Department of Internal Medicine, Kainan Municipal Medical Center
  • HIROI Takayuki
    Department of Hematology/Oncology, Wakayama Medical University Department of Hematology, Kinan Hospital
  • YAMASHITA Yusuke
    Department of Hematology/Oncology, Wakayama Medical University Department of Hematology, Kinan Hospital
  • OIWA Takehiro
    Department of Hematology/Oncology, Wakayama Medical University
  • MURATA Shogo
    Department of Hematology/Oncology, Wakayama Medical University
  • MUSHINO Toshiki
    Department of Hematology/Oncology, Wakayama Medical University Department of Internal Medicine, Kainan Municipal Medical Center
  • NISHIKAWA Akinori
    Department of Hematology/Oncology, Wakayama Medical University
  • HANAOKA Nobuyoshi
    Department of Hematology/Oncology, Wakayama Medical University Department of General Medicine, National Hospital Organization Kumamotominami National Hospital
  • SONOKI Takashi
    Department of Hematology/Oncology, Wakayama Medical University

Bibliographic Information

Other Title
  • 和歌山県における発作性夜間ヘモグロビン尿症に対するeculizumab療法の後方視的検討
  • 臨床研究 和歌山県における発作性夜間ヘモグロビン尿症に対するeculizumab療法の後方視的検討
  • リンショウ ケンキュウ ワカヤマケン ニ オケル ホッサセイ ヤカン ヘモグロビン ニョウショウ ニ タイスル eculizumab リョウホウ ノ コウホウ シテキ ケントウ

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<p>Currently, the humanized anti-C5 monoclonal antibody, eculizumab, is widely used for treating paroxysmal nocturnal hemoglobinuria (PNH) due to its effects on suppression of intravascular hemolysis and resulting improvement in quality of life. However, in some cases, this treatment is refractory or is associated with meningococcal meningitis. No region-specific analyses have been published, and currently, information on region specificity and genetic factors is limited. We present here the results of a retrospective study involving eight patients with PNH who were treated with eculizumab in our hospital in Wakayama, Japan. The median age of these patients was 77 (range 23-88) years. Six patients had a complication of aplastic anemia, four patients had a history of thrombosis, and two experienced hemolytic episodes. Before initiating eculizumab treatment, the median serum LDH level was 1,192 IU/l (range 755-1,525 IU/l). Serum LDH levels normalized in five patients within a month of initiating therapy and PNH-related symptoms disappeared. C5 gene mutations were identified in the three patients who did not respond to eculizumab.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 61 (6), 605-611, 2020

    The Japanese Society of Hematology

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